摘要
纤维连接蛋白肾小球疾病(FNG)是一种罕见的常染色体显性遗传性疾病,以大量纤维连接蛋白沉积于肾小球内皮下及系膜区为其特征,临床表现为蛋白尿、不同程度的血尿、高血压及进展缓慢的肾功能减退,少数患者有肾小管酸中毒。其诊断主要依据肾穿刺结果纤维连接蛋白荧光染色阳性,目前无特异性治疗,多给予对症治疗。FNG发病罕见,合并具有肾脏意义的单克隆免疫球蛋白血症(MGRS)极少报道。本文报道1例FNG合并MGRS,并进行相关文献的复习。
Fibronect in glomerular disease(FNG)is a rare autosomal dominant in herited disease characterized by a large amount of fibronectin deposited in the glomerular subendothelial and mesangium.The clinical manifestations are proteinuria,a different level of hematuria,hypertension,and slowly progressing renal dysfunction.And a small number of patients have renal tubular acidosis.The diagnosis is mainly based on the results of renal puncture,and the fibronectin staining is positive.There is no specific treatment,and more symptomatic treatment should be used.FNG is rare,and the complication,monoclonal immunoglobulinemia(MGRS)with renal significance,is rarely reported.This article reported a case of FNG accompanied by MGRS and literature were reviewed.
作者
朱敏敏
马志刚
Zhu Minmin;Ma Zhigang(College of Clinical Medicine,Gansu University of Chinese Medicine,Lanzhou 730000,China;Department of Nephrology,Gansu Provincial Hospital,Lanzhou 730000,China)
出处
《临床荟萃》
CAS
2019年第11期1018-1021,共4页
Clinical Focus
